肾脏最大径大于4 cm的大体积上皮样血管平滑肌脂肪瘤临床特征分析  

Clinical analysis of large-volume renal epithelioid angiomyolipoma(maximum diameter>4 cm)

作　　者：张俊[1] 郁文谊 金李晨 侯建全[1,2] ZHANG Jun;YU Wenyi;JIN Lichen;HOU Jianquan(Department of Urology,the First Affliated Hospital of Soochow University,Jiangsu Suzhou 215000,China;Department of Urology,Dushu Lake Hospital Affiliated to Soochow University,Jiangsu Suzhou 215000,China)

机构地区：[1]苏州大学附属第一医院泌尿外科,江苏苏州215000 [2]苏州大学附属独墅湖医院泌尿外科,江苏苏州215000

出　　处：《现代肿瘤医学》2024年第16期3077-3084,共8页Journal of Modern Oncology

摘　　要：目的:分析大体积(最大径>4 cm)肾脏上皮样血管平滑肌脂肪瘤(EAML)的临床特征。方法:纳入2010至2021年在我院诊断为肾脏EAML,且符合纳入标准的30例患者,分析其临床、病理和治疗特点。结果:30例受试者中,男性12例,女性18例,年龄26~74岁。所有患者均行CT扫描。大多数EAML患者(23/30)在平扫CT图像上表现为相对于肾实质的高密度及以高密度为主的混杂密度,7例表现为低密度。完全性EAML的患者相对于部分EAML的患者,其肿块在平扫CT上表现为更大面积的高密度影。肿瘤最大径(8.7±6.6)(4.0~27.7)cm。所有患者均接受手术治疗并获得随访,随访时间24~150个月,中位随访时间59.5个月。随访患者中,1例死于肿瘤恶液质并伴有肝转移,3例出现同侧复发,1例患者出现肝转移,均为完全性EAML。结论:上皮样血管平滑肌脂肪瘤(EAML)是一种相对罕见的乏脂肪肾肿瘤。相对于小体积的EAML存在典型的CT表现,大体积的EAML在平扫CT上高密度特征已不再典型。只有少数EAML具有恶性潜能。手术治疗效果较好,但术后可能会出现复发和转移,尤其是老年患者,以及完全性EAML患者,建议密切随访。Objective:To investigate the clinical characteristics of large-volume epithelioid angiomyolipoma(EAML)(maximum diameter>4 cm)of the kidney.Methods:This study included 30 patients who were diagnosed with renal large-volume EAML in our hospital from 2010 to 2021 and met the inclusion criteria,and analyzed their clinical,pathological,and therapeutic characteristics.Results:Among the 30 subjects,there were 12 males and 18 females,aged 26~74 years.All patients underwent CT scan.Most EAML patients(23/30)showed hyper-attenuation and mixed-attenuation with hyper-attenuation relative to renal parenchyma on unenhanced CT images,and 7 cases showed hypo-attenuation.Compared with patients with partial EAML,patients with pure EAML showed a larger area of hyper-attenuation on unenhanced CT.The maximum diameter of the tumor was(8.7±6.6)(4.0~27.7)cm.All patients underwent surgical treatment and were followed up for 24~150 months,with a median follow-up time of 59.5 months.Among the follow-up patients,1 patient died of tumor cachexia with liver metastasis,3 patients had ipsilateral recurrence,and 1 patient had liver metastasis,all of which were pure EAML.Conclusion:EAML is a relatively rare renal angiomyolipoma with minimal fat.Compared with the small volume of EAML,there are typical CT manifestations,and the hyper-attenuation characteristics of large volume of EAML on unenhanced CT are no longer typical.Most EAMLs do not show a tendency to progress,and only a few have malignant potential.However,recurrence and metastasis may occur after surgery,especially in elderly patients and patients with pure EAML,and close follow-up is recommended.

关 键 词：EAML 上皮样血管平滑肌脂肪瘤 乏脂型AML 完全性EAML 肾肿瘤 

分 类 号：R737.11[医药卫生—肿瘤]